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Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS

Expansion of a (G(4)C(2))(n) repeat in C9orf72 causes amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), but the link of the five repeat-encoded dipeptide repeat (DPR) proteins to neuroinflammation, TDP-43 pathology, and neurodegeneration is unclear. Poly-PR is most toxic in vitr...

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Detalles Bibliográficos
Autores principales:	LaClair, Katherine D., Zhou, Qihui, Michaelsen, Meike, Wefers, Benedikt, Brill, Monika S., Janjic, Aleksandar, Rathkolb, Birgit, Farny, Daniel, Cygan, Mikolaj, de Angelis, Martin Hrabe, Wurst, Wolfgang, Neumann, Manuela, Enard, Wolfgang, Misgeld, Thomas, Arzberger, Thomas, Edbauer, Dieter
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Springer Berlin Heidelberg 2020
Materias:	Original Paper
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7360660/ https://www.ncbi.nlm.nih.gov/pubmed/32562018 http://dx.doi.org/10.1007/s00401-020-02176-0

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